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The controversy of adjuvant radiotherapy of meningiomas is at least partially due to the insufficient understanding on meningioma cells' response to irradiation and the shortage of radiosensitivity-promotion methods. MicroRNA-221 and microRNA-222 were identified as critical regulators of radiosensitivity in several other tumors. However, their effect in meningiomas has yet to be confirmed. Therefore, the malignant meningioma IOMM-Lee cells were adopted, transfected with microRNA-221/222 mimics or inhibitors, and irradiated with different dosages. The effects of radiation and microRNA-221/222 were then assessed in vitro and in vivo. Radiation dose increases and microRNA-221/222 downregulation synergistically inhibited cell proliferation and colony formation, prevented xenograft tumor progression, and promoted apoptosis, but antagonistically regulated cell invasiveness. Pairwise comparisons revealed that only high-dose radiations (6 and 8 Gy) can significantly promote cell invasiveness in comparison with unirradiated counterparts. Further comparisons exhibited that downregulating the microRNA-221/222 expression can reverse this radiation-induced cell invasiveness to a level of untransfected and unirradiated cells only if cells were irradiated with no more than 6 Gy. In addition, this approach can promote IOMM-Lee's radiosensitivity. Meanwhile, we also detected that the dose rate of irradiation affects cell cycle distribution and cell apoptosis of IOMM-Lee. A high dose rate irradiation induces G0/G1 cell cycle arrest and apoptosis-promoting effect. Therefore, for malignant meningiomas, high-dose irradiation can facilitate cell invasiveness significantly. Downregulating the microRNA-221/222 level can reverse the radiation-induced cell invasiveness while enhancing the apoptosis-promoting and proliferation-inhibiting effects of radiation and promoting cell radiosensitivity.
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BACKGROUND: Intracranial fibrosarcoma is an extremely rare neoplasm in the central nervous system. Insofar there were only sporadic case reports describing its features. The purpose of this study is to review the clinical and surgical features of cases who were treated in our department. METHOD: The authors retrospectively reviewed and detailed the clinical and surgical data obtained from 5 patients with fibrosarcoma who underwent treatment at our institute between January 2009 and January 2019. RESULTS: There were 3 males and 2 females including 2 juvenile and 3 senior patients. The most frequent sign was intermittent pain and vomiting. The location of the tumor included middle fossa, thalamus and midbrain, sellar and suprasellar region and right parietal-occipital lobe. Surgical observation demonstrated the consistency of the tumor was tenacious with abundant blood supply. Gross total resection was achieved in 2 cases. Pathological analysis showed spindle cells in a herringbone form with positive Vimentin staining in all 5 cases, with the absence of GFAP or S-100. All 5 patients were deceased eventually after a varied period of time after the first surgery. CONCLUSION: Intracranial fibrosarcoma was a highly malignant entity presented in the central nervous system. Surgery still remains the first-line treatment followed by radiotherapy, however, the prognostic outcome was very poor. Future studies should be more focused on accumulation of the relevant information on this disease thus hopefully in assisting to developing more optimized treatment.
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Fibrossarcoma/diagnóstico por imagem , Neoplasias Cranianas/diagnóstico por imagem , Adolescente , Adulto , Idoso , Criança , Feminino , Fibrossarcoma/metabolismo , Fibrossarcoma/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Lobo Occipital/diagnóstico por imagem , Lobo Occipital/metabolismo , Lobo Occipital/cirurgia , Prognóstico , Estudos Retrospectivos , Neoplasias Cranianas/metabolismo , Neoplasias Cranianas/cirurgia , Vimentina/metabolismo , Adulto JovemRESUMO
BACKGROUND: Ocular motor dysfunction is one of the most common postoperative complications of petroclival meningioma. However, its incidence, recovery rate, and independent risk factors remain poorly explored. METHODS: A prospective analysis of 31 petroclival meningiomas was performed. Operative approaches were selected by utilizing a new 6-region classification of petroclival meningiomas we proposed. Two scores were used to evaluate the functions of the oculomotor and abducens nerves. Pearson correlation analysis and binary logistic regression analysis were used to identify independent risk factors for intraoperative oculomotor and abducens nerve injury. RESULTS: Postoperative new-onset dysfunctions in the pupillary light reflex and eye/eyelid movements as well as abducens paralysis were detected in eight (25.8%), ten (32.3%) and twelve (38.7%) cases, respectively. Their corresponding recovery rates after 6 months of follow-up were 75% (6/8), 80% (8/10), and 83.3% (10/12), respectively, and their mean times to start recovery were 4.03, 2.43, and 2.5 months, respectively. Tumor invasion into the suprasellar region/sphenoid sinus was the only risk factor for dysfunctions in both the pupillary light reflex (p = 0.001) and eye/eyelid movements (p = 0.002). Intraoperative utilization of the infratrigeminal interspace was the only risk factor for dysfunction in eyeball abduction movement (p = 0.004). CONCLUSIONS: Dysfunctions of the oculomotor and abducens nerves recovered within 6 months postoperatively. Tumor extension into the suprasellar region/sphenoid sinus was the only risk factor for oculomotor nerve paralysis. Eye/eyelid movements were more sensitive than the pupillary light reflex in reflecting nerve dysfunctions. Intraoperative utilization of the infratrigeminal interspace was the only risk factor for abducens nerve paralysis.
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Neoplasias Meníngeas/cirurgia , Meningioma/cirurgia , Procedimentos Neurocirúrgicos/efeitos adversos , Traumatismos do Nervo Oculomotor/fisiopatologia , Complicações Pós-Operatórias/fisiopatologia , Neoplasias da Base do Crânio/cirurgia , Nervo Abducente/patologia , Traumatismo do Nervo Abducente/etiologia , Traumatismo do Nervo Abducente/fisiopatologia , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Procedimentos Neurocirúrgicos/métodos , Nervo Oculomotor/patologia , Traumatismos do Nervo Oculomotor/etiologia , Complicações Pós-Operatórias/etiologia , Reflexo PupilarRESUMO
OBJECTIVE: To investigate the independent risk factors for recurrence in intracranial atypical meningiomas (AMs) treated with gross total resection (GTR) and early external beam radiotherapy (EBRT). METHODS: Clinical, radiological, and pathological data of intracranial AMs treated with GTR-plus-early-EBRT between January 2008 and July 2016 were reviewed. Immunohistochemical staining for Ki-67 was performed. Kaplan-Meier curves and univariate and multivariate Cox proportional hazards regression analyses were used to explore independent predictors of tumor recurrence. Chi square test was performed to compare variables between subgroups. RESULTS: Forty-six patients with intracranial AMs underwent GTR and early EBRT. Ten (21.7%) recurred and three (6.5%) died during a median follow-up of 76.00 months. Univariate and multivariate Cox analyses revealed that malignant progression (MP) (P = 0.009) was the only independent predictor for recurrence, while Ki-67 was of minor value in this aspect (P = 0.362). MP-AMs had a significantly higher recurrence rate (P = 0.008), a higher proportion of irregularly shaped tumors (P = 0.013) and significantly lower preoperative Karnofsky Performance Scale (KPS) scores (P = 0.040) than primary (Pri) AMs. No significant difference in Ki-67 expression was detected between these subgroups (P = 0.713). CONCLUSIONS: MP was significantly correlated with an increased incidence of recurrence in GTR-plus-early-EBRT-treated intracranial AMs. Significantly higher frequencies of tumor relapse and irregularly shaped tumors and lower preoperative KPS scores were observed in MP-AMs compared with Pri-AMs. Ki-67 expression is of minor value in predicting tumor recurrence or distinguishing tumor origins in AMs.
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OBJECTIVE: To develop a method to distinguish atypical meningiomas (AMs) with malignant progression (MP) from primary AMs without a clinical history. METHODS: The clinical, radiologic, and pathologic data of 33 previously Simpson grade I resected (if any) as well as no radiotherapy treated intracranial AMs between January 2008 and December 2015 were reviewed. Immunohistochemical staining for connexin 43 (Cx43) and Ki-67 was performed. Descriptive analysis and univariate and multivariate logistic regression analyses were used to explore independent predictors of MP. A multivariable logistic model was developed to estimate the risk of MP, and its diagnostic value was determined from a receiver operating characteristic curve. RESULTS: There were 11 AMs (33.3%) with histopathologically confirmed MP from benign meningiomas. The other 22 (66.7%) were initially diagnosed AMs with no histopathologically confirmed MP during a median 60.5 months (range, 42-126 months) of follow-up. Univariate and multivariate logistic analyses showed that irregular tumor shape (P = 0.010) and low Cx43 expression (P = 0.010) were independent predictors of the presence of MP, and the predicted probability was calculated by the following formula: P = 1/[1+exp.{1.218-(3.202×Shape)+(3.814×Cx43)}]. P > 0.5 for an irregularly shaped (score 1) AM with low Cx43 expression (score 0) indicated a high probability of MP. The sensitivity, specificity, positive predictive value, negative predictive value, and overall predictive accuracy were 63.6, 95.6, 87.5, 84.0, and 84.8%, respectively. CONCLUSIONS: Low Cx43 expression and irregular tumor shape were independent predictors of the presence of MP. The relevant logistic regression model was found to be effective in distinguishing MP-AMs from primary AMs.
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Modelos Logísticos , Neoplasias Meníngeas/patologia , Meningioma/patologia , Adulto , Idoso , Biomarcadores Tumorais/análise , Conexina 43/biossíntese , Progressão da Doença , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
OBJECTIVES: Limited data regarding intracranial mesenchymal chondrosarcoma (MCS) are available. The goal of this study was to report the clinical characteristics, challenges in management, and poor outcomes of intracranial MCS. METHODS: Clinical data for 16 patients with MCS were reviewed retrospectively to evaluate their clinical characteristics, management, and outcomes. RESULTS: This study included 11 male and 5 female patients with a mean age of 22.9 ± 14.4 years. The most common presentations were headache (n = 10; 62.5%), followed by cranial deficits (n = 7; 43.6%). The radiologic spectrum for MCS was broad, and only 18.8% (3/16) of MCSs were correctly diagnosed preoperatively. Aggressive resection (including subtotal resection and gross total resection) and partial resection was performed in 62.5% (10/16) and 37.50% (6/16) of patients. With a median follow-up of 34 months (range, 10-78 months), 5 patients (31.3%) died and 8 patients (50%) developed tumor recurrence. The 1-, 3-, and 5-year rates of progression-free survival and overall survival were 86%, 53%, and 42% and 93%, 70%, and 56%, respectively. Although the differences were not significantly different, aggressive resection and the use of radiotherapy tended to improve the prognosis of the patients. CONCLUSIONS: Clinical characteristics of MCS are variable. The current management of intracranial MCS referring to conventional chondrosarcoma could not yield satisfactory outcomes. Further study is needed to identify the optimal treatments.
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Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/terapia , Condrossarcoma Mesenquimal/diagnóstico por imagem , Condrossarcoma Mesenquimal/terapia , Adolescente , Adulto , Neoplasias Ósseas/mortalidade , Criança , Pré-Escolar , Condrossarcoma Mesenquimal/mortalidade , Condrossarcoma Mesenquimal/cirurgia , Feminino , Humanos , Estimativa de Kaplan-Meier , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Índice de Gravidade de Doença , Tomógrafos Computadorizados , Adulto JovemRESUMO
BACKGROUND: Subependymoma is rare, and little is known about subependymoma with intratumoral hemorrhage. METHODS: A retrospective study of subependymoma was performed. Among 61 subependymomas, 4 cases of intratumoral hemorrhage were collected. All 4 cases were pathologically confirmed to be subependymoma and showed a benign character. RESULTS: After complete subependymoma resection, the 4 patients achieved favorable outcomes. Pathology showed that dilated thin-walled vessels and/or hyalinosis of the vessel walls existed in all 4 cases. CONCLUSIONS: The present series showed that subependymomas with hemorrhage and benign pathology are rare and that surgical treatment results in good prognosis. This series supports the hypothesis that the pathology of vascular degeneration may contribute to subependymoma hemorrhage.
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Encéfalo/diagnóstico por imagem , Hemorragia Cerebral/cirurgia , Neoplasias do Ventrículo Cerebral/cirurgia , Glioma Subependimal/patologia , Glioma Subependimal/cirurgia , Adulto , Hemorragia Cerebral/etiologia , Feminino , Glioma Subependimal/complicações , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento , Adulto JovemRESUMO
OBJECTIVE: Primary intracranial angioleiomyoma is a rare and distinct neoplasm. Only 29 cases have been reported previously, and we aimed to investigate the clinical and radiopathologic features of these lesions. METHODS: Medical records and radiographs of 8 patients (7 male and 1 female; mean age: 48.7 years) at our institution were reviewed retrospectively. Patient follow-up and a literature review were performed. RESULTS: The most common preoperative symptom was a visual defect (n = 2), followed by diplopia (n = 1) and abducens paralysis (n = 1). Three patients were asymptomatic. The parasellar area (particularly the cavernous sinus) was the predilection site (n = 4; 50.0%). Radiographically, all lesions were solid without cystic degeneration. All lesions appeared with T1 hypointensity and T2 hyperintensity, and they were gradually heterogeneously enhanced after the administration of gadolinium. Complete resection was achieved in 7 patients (87.5%) without recurrence after 26.8 months of follow-up. Mitosis was rarely observed, and the Ki-67 labeling index was less than 1%; pathologically, the cavernous type was the most common. CONCLUSIONS: Primary intracranial angioleiomyomas were prevalent in middle-aged men, and they usually involved the cavernous sinus and were frequently pathologically identified as the cavernous type. Preoperative symptoms varied depending on lesion location. The preoperative diagnosis of primary intracranial angioleiomyomas is difficult without pathology. Digital subtraction angiography and preoperative embolization are useful for differential diagnosis and surgery. Given the indolent biology of these tumors, a favorable outcome can be achieved using total resection without recurrence. A larger sample size with long-term follow-up is needed to verify our findings.
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Angiomioma/diagnóstico por imagem , Angiomioma/cirurgia , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/cirurgia , Adulto , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
The impact of sexual dysfunction (SD) is distressing to many male patients with pituitary adenomas which affect both physical and psychological health. The research explored to identify risk factors affecting sexual function and the prognosis of male patients with pituitary adenomas. Two hundred and fifty-four male patients, who aged between 18 and 60 (mean ± s.d.: 44.16 ± 10.14) years and diagnosed with pituitary adenomas, were retrospectively analyzed. One hundred and fifty-nine patients (62.6%) complained of SD prior to surgery. The mean International Index of Erectile Function (IIEF-5) in patients with giant adenomas was 16.13 ± 2.51, much smaller than those with microadenomas or macroadenomas (P < 0.05). All the patients showed significant improvement in terms of erectile dysfunction (ED) following surgery (P < 0.05). In addition, complete resection achieved a higher degree of SD relief than partial resection. The incidence of SD in functioning pituitary adenomas (FPAs) was much higher than that in nonfunctioning pituitary adenomas (NFPAs) (P < 0.05). In addition, compared with NFPAs, males with prolactinomas (82.8%) had the higher prevalence of SD and significantly improvement following surgical intervention (P < 0.05). An inverse relationship was identified between decreasing testosterone levels and increasing incidence of SD before surgery (P < 0.05). There was no significant difference between 6 months and 12 months after surgery in serum testosterone level (P > 0.05). Our results indicated that surgical therapy could be optimized for improvements in SD and that testosterone levels can be used as a sensitive indicator to predict the recovery rate of sexual function in patients with pituitary adenomas following surgery and the serum testosterone level will stay stable in 6 months after surgery.
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Adenoma/complicações , Neoplasias Hipofisárias/complicações , Disfunções Sexuais Fisiológicas/etiologia , Adenoma/patologia , Adenoma/cirurgia , Adolescente , Adulto , Estudos de Coortes , Disfunção Erétil/epidemiologia , Disfunção Erétil/etiologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Neoplasias Hipofisárias/patologia , Neoplasias Hipofisárias/cirurgia , Valor Preditivo dos Testes , Prognóstico , Prolactinoma/complicações , Prolactinoma/cirurgia , Estudos Retrospectivos , Fatores de Risco , Disfunções Sexuais Fisiológicas/epidemiologia , Testosterona/sangue , Resultado do Tratamento , Adulto JovemRESUMO
BACKGROUND: Brainstem intramedullary schwannomas (ISs) are extremely rare. Various theories have been suggested to explain its origin. It was first speculated that ISs arise from the region where the nerve roots lose their sheaths on penetrating the pia mater. Later, it was further predicted that ISs would contain both intra- and extramedullary parts and would be shaped like a dumbbell. However, no cases reported previously can support this assumption adequately. CASE DESCRIPTION: A 40-year-old woman presented with constant cervical pain, accompanied by progressive weakness of upper extremities and glove distribution numbness. Magnetic resonance imaging of the brain revealed a rare intra- and extramedullary dumbbell-shaped lesion of the medulla oblongata, which was partially removed via a midline suboccipital craniectomy. Histologic and immunohistochemical examinations confirmed the diagnosis of schwannoma. Routine imaging performed 20 months after the initial resection revealed a regrowth of the intramedullary part, which was subsequently partially removed through a far-lateral approach, with symptoms alleviated. At 2-year follow-up, there continued to be no radiologic or clinical evidence of regrowth. CONCLUSIONS: To date and to our knowledge, there are only 16 reported cases of brainstem ISs, none of which contained both intra- and extramedullary components. We believe this is the first report of dumbbell schwannoma of the medulla oblongata with adequate radiologic evidence. The relevant literature is reviewed, and an assumption has been proposed that dumbbell or surfacing ISs arising near entry zones of sensory nerves, mixed cranial nerves, or ventral root may originate from the aberrant Schwann cells.
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Neoplasias Encefálicas/patologia , Bulbo/patologia , Neurilemoma/patologia , Adulto , Antígenos CD34/metabolismo , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/cirurgia , Feminino , Humanos , Imageamento por Ressonância Magnética , Bulbo/diagnóstico por imagem , Bulbo/metabolismo , Bulbo/cirurgia , Neurilemoma/diagnóstico por imagem , Neurilemoma/cirurgia , Proteínas S100/metabolismoRESUMO
BACKGROUND: Over the years, the mechanical ventilation (MV) strategy has changed worldwide. The aim of the present study was to describe the ventilation practices, particularly lung-protective ventilation (LPV), among brain-injured patients in China. METHODS: This study was a multicenter, 1-day, cross-sectional study in 47 Intensive Care Units (ICUs) across China. Mechanically ventilated patients (18 years and older) with brain injury in a participating ICU during the time of the study, including traumatic brain injury, stroke, postoperation with intracranial tumor, hypoxic-ischemic encephalopathy, intracranial infection, and idiopathic epilepsy, were enrolled. Demographic data, primary diagnoses, indications for MV, MV modes and settings, and prognoses on the 60th day were collected. Multivariable logistic analysis was used to assess factors that might affect the use of LPV. RESULTS: A total of 104 patients were enrolled in the present study, 87 (83.7%) of whom were identified with severe brain injury based on a Glasgow Coma Scale ≤8 points. Synchronized intermittent mandatory ventilation (SIMV) was the most frequent ventilator mode, accounting for 46.2% of the entire cohort. The median tidal volume was set to 8.0 ml/kg (interquartile range [IQR], 7.0-8.9 ml/kg) of the predicted body weight; 50 (48.1%) patients received LPV. The median positive end-expiratory pressure (PEEP) was set to 5 cmH2O (IQR, 5-6 cmH2O). No PEEP values were higher than 10 cmH2O. Compared with partially mandatory ventilation, supportive and spontaneous ventilation practices were associated with LPV. There were no significant differences in mortality and MV duration between patients subjected to LPV and those were not. CONCLUSIONS: Among brain-injured patients in China, SIMV was the most frequent ventilation mode. Nearly one-half of the brain-injured patients received LPV. Patients under supportive and spontaneous ventilation were more likely to receive LPV. TRIAL REGISTRATION: ClinicalTrials.org NCT02517073 https://clinicaltrials.gov/ct2/show/NCT02517073.
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Lesões Encefálicas/terapia , Respiração Artificial , Adulto , Idoso , Lesões Encefálicas Traumáticas/terapia , China , Estudos Transversais , Feminino , Humanos , Hipóxia-Isquemia Encefálica/terapia , Unidades de Terapia Intensiva/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Acidente Vascular Cerebral/terapia , Inquéritos e QuestionáriosRESUMO
OBJECTIVE The aim of this study was to analyze the neurological functional outcome and recurrent risks in surgically treated jugular foramen paragangliomas (JFPs) and to propose an individualized therapeutic strategy. METHODS Clinical charts and radiological information were reviewed retrospectively in 51 consecutive cases of JFPs. Less-aggressive surgical interventions were adopted with the goal of preserving neurovascular structures. Scheduled follow-up was performed. RESULTS The mean age of the patients in the cases reviewed was 41.6 years, and the group included 27 females (52.9%). The mean preoperative Karnofsky Performance Scale (KPS) score was 78.4. The mean lesion size was 3.8 cm. Forty-three cases (84.3%) were Fisch Type D, including 37 cases (72.5%) of Type Di1 and Di2. Thirty-seven cases (72.5%) were Glasscock-Jackson Type III-IV. Gross-total resection and subtotal resection were achieved in 26 (51.0%) and 22 (43.1%) cases, respectively. Surgical morbidities occurred in 23 patients (45.1%), without surgery-related mortality after the first operation. The mean postoperative KPS scores at discharge, 3 months, 1 year, and most recent evaluation were 71.8, 77.2, 83.2, and 79.6, respectively. The mean follow-up duration was 85.7 months. The tumor recurrence/regrowth (R/R) rate was 11.8%. Compared with preoperative status, swallowing function improved or stabilized in 96.1% and facial function improved or stabilized in 94.1% of patients. A House-Brackmann scale Grade I/II was achieved in 43 patients (84.3%). Overall neurological status improved or stabilized in 90.0% of patients. Pathological mitosis (HR 10.640, p = 0.009) was the most significant risk for tumor R/R. A 1-year increase in age (OR 1.115, p = 0.037) and preoperative KPS score < 80 (OR 11.071, p = 0.018) indicated a risk for recent poor neurological function (KPS < 80). Overall R/R-free survival, symptom progression-free survival, and overall survival at 15 years were 78.9%, 86.8%, and 80.6%, respectively. CONCLUSIONS Surgical outcomes for JFPs were acceptable using a less-aggressive surgical strategy. Most patients could adapt to surgical morbidities and carry out normal life activities. Preserving neurological function was a priority, and maximal decompression with or without radiotherapy was desirable to preserve a patient's quality of life when radical resection was not warranted. Early surgery plus preoperative devascularization was proposed, and radiotherapy was mandatory for lesions with pathological mitosis.
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Procedimentos Neurocirúrgicos/métodos , Paraganglioma/cirurgia , Neoplasias Cranianas/cirurgia , Osso Temporal , Adulto , Feminino , Humanos , Masculino , Estudos Retrospectivos , Fatores de Tempo , Resultado do TratamentoRESUMO
OBJECTIVE: To investigate the outcomes of jugular foramen schwannomas (JFSs) and to evaluate the risk factors for tumor recurrence and poor final outcomes. METHODS: Between 1993 and 2013, 133 patients (68 female patients) with JFSs were surgically treated. Clinical charts were reviewed, and scheduled follow-up examinations were performed. RESULTS: The average preoperative Karnofsky Performance Scale (KPS) score was 79.6. The JFSs were classified as follows: 65 cases, type A; 15 cases, type B; 5 cases, type C; and 48 cases, type D. Gross total resection was achieved in 107 (80.5%) patients. Transient and permanent morbidities affecting cranial nerves IX and X were 19.8% and 11.5%, respectively. After a mean follow-up duration of 108.0 months, 13 (9.9%) patients experienced recurrence. The most recent KPS scores averaged 83.7. Compared with the preoperative KPS score, the most recent KPS score was improved in 87 (65.4%) patients and stabilized in 29 (21.8%) patients. The presence of a solid tumor (hazard ratio [HR] = 5.815, P = 0.010), nontotal resection (HR = 4.613, P = 0.007), and pathologic mitoses (HR = 11.018, P < 0.001) were independent risk factors for tumor recurrence. Decreased preoperative KPS score (per 10 points) (odds ratio [OR] = 2.483, P = 0.027), a less soft tumor consistency (OR = 2.257, P = 0.039), and a solid tumor (OR = 3.755, P = 0.041) were risk factors for poor long-term outcomes. CONCLUSIONS: Quality of life and preservation of neurologic function are the goals of surgical treatment of JFSs. Favorable long-term surgical outcomes for JFSs can be achieved. Morbidity of cranial nerves IX and X is significant, and patients with nontotal resection or pathologic mitosis should be followed closely.
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Neoplasias Encefálicas/cirurgia , Recidiva Local de Neoplasia/epidemiologia , Neurilemoma/cirurgia , Complicações Pós-Operatórias , Adolescente , Adulto , Idoso , Neoplasias Encefálicas/complicações , Neoplasias Encefálicas/mortalidade , Criança , Feminino , Humanos , Avaliação de Estado de Karnofsky , Masculino , Pessoa de Meia-Idade , Neurilemoma/complicações , Neurilemoma/mortalidade , Qualidade de Vida , Recuperação de Função Fisiológica , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Adulto JovemRESUMO
Von Hippel-Lindau (VHL) disease is an autosomal dominantly inherited neoplastic disorder characterized by marked phenotypic variability and age-dependent penetrance. This disease is caused by germline mutations in the VHL tumor suppressor gene. Systematic physical examinations, imaging assessments and molecular genetic tests for the VHL gene were performed in a large Chinese VHL family. The examined Chinese VHL family, which has 25 members from four generations, including 7 diagnosed VHL patients and 2 asymptomatic mutation carriers. The average ages of first onset for generations I, II, and III were 37, 30 and 16, respectively. The male:female ratio among VHL patients was 6:1. Molecular genetic investigations detected the c.433C>T [p.Q145X] nonsense mutation in the VHL gene. Molecular modeling of the VHL-ElonginC- ElonginB-HIF-1α complex predicted that the p.Q145X mutation markedly alters the L7 loop structure of the ß-domain of the VHL protein (pVHL), destabilizes the VHL-HIF-1αcomplex, and induces the truncation of pVHL. We speculate that the p.Q145X nonsense mutation leads to relatively obvious familial aggregation. This mutation causes the type I phenotype of VHL disease and is associated with a high risk of retinal and central nervous system (CNS) hemangioblastomas (HGBs) and visceral cysts, but a low risk of renal cell carcinoma. Moreover, within a VHL family, the average ages of first onset became younger in successive generations, and the CNS HGBs are more likely to occur in male patients.
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Códon sem Sentido/genética , Proteína Supressora de Tumor Von Hippel-Lindau/genética , Doença de von Hippel-Lindau/genética , Adolescente , Adulto , Animais , Povo Asiático , Encéfalo/patologia , Análise Mutacional de DNA , Saúde da Família , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Medula Espinal/patologia , Proteína Supressora de Tumor Von Hippel-Lindau/química , Adulto Jovem , Doença de von Hippel-Lindau/patologiaRESUMO
This study seeks to elucidate the prognostic predictors and outcomes of recurrent/progressive petroclival meningiomas (PCMs). We reviewed our cohort of 39 recurrent/progressive PCMs (27 females, 69.2%) and analyzed the results from the literature. Twenty-three patients underwent reoperations, 2 received radiotherapy alone, and 14 declined any treatment. During a follow-up of 70.4 months, 7 patients experienced a 2nd recurrence/progression (R/P) and 18 patients died. In the 23 patients, gross total resection (GTR), subtotal resection (STR), and partial resection (PR) were achieved in 8, 8, and 7 patients, respectively. The percentage of the 2nd R/P-free survival of GTR, STR, and PR was 88%, 67%, and 40%, respectively. The overall survival following the 1st R/P of GTR, STR, and PR was 88%, 63%, and 33%, respectively. Patients rejecting treatment suffered from significantly poor overall survival (7%; p = 0.001) and short survival duration (42.0 months; p = 0.016) compared with that of the patients receiving treatment (67% and 86.9 months). The GTR was the only independent favorable predictor. In the 21 included studies with 98 recurrent/progressive PCM patients, 17 patients presented with a 2nd R/P and 10 died of a 2nd R/P; patients undergoing observation had a significantly poor tumor regrowth control rate compared with patients undergoing surgery (p = 0.004) or radiotherapy alone (p < 0.001). Proactive treatment should be performed for patients with recurrent/progressive PCMs. Observation can lead to relentless outcome. GTR as a preferential therapeutic strategy should be pursued as far as possible on the condition of minimal functional impairment.
Assuntos
Neoplasias Meníngeas/diagnóstico , Meningioma/diagnóstico , Recidiva Local de Neoplasia/diagnóstico , Neoplasias da Base do Crânio/diagnóstico , Adulto , Idoso , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Neoplasias Meníngeas/cirurgia , Meningioma/cirurgia , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/cirurgia , Procedimentos Neurocirúrgicos/métodos , Prognóstico , Neoplasias da Base do Crânio/patologia , Neoplasias da Base do Crânio/cirurgia , Resultado do TratamentoRESUMO
UNLABELLED: OBJECT.: Cerebral cavernous malformations have been studied widely, but the natural history of brainstem cavernous malformations (CMs) is not well defined, and hemorrhages caused by brainstem CMs are devastating. The goal of this study was to quantify the hemorrhage risks and functional outcomes of patients with brainstem CMs. METHODS: This prospective, longitudinal, cohort study included patients with brainstem CMs diagnosed between 1985 and 2012. The clinical courses of all patients were recorded. Predictors of hemorrhage and the overall untreated outcomes were evaluated. RESULTS: A total of 331 patients (46.5% female) were included, with a mean follow-up duration of 6.5 years. The annual hemorrhage rates in patients initially presenting with hemorrhage with (n = 215) or without (n = 34) focal neurological deficits were 15.9% and 12.4%, respectively. However, the annual hemorrhage rate was 8.7% in patients initially presenting without hemorrhage (n = 82). The risk factors for hemorrhage were female sex (hazard ratio [HR] 1.445, p = 0.041), prior hemorrhage (HR 1.277, p = 0.029), and perilesional edema (HR 1.830, p = 0.002). Overall, neurological function at the most recent assessment was improved compared with neurological function at diagnosis. Additionally, 307 patients (92.7%) improved or stabilized, 268 (81.0%) lived independently, and 95 (28.7%) completely recovered. Predictors favoring complete recovery were no prospective hemorrhage (HR 1.958, p = 0.001), younger age (HR 1.268, p = 0.001), and small lesion size (HR 1.578, p = 0.004). CONCLUSIONS: Patients' initial presentation predicts their prospective annual hemorrhage rate. This study suggests that several strong risk factors for hemorrhage and predictors of brainstem CM outcomes may enable clinicians to evaluate the potential hemorrhage risks of their patients and design personalized treatments.
Assuntos
Neoplasias do Tronco Encefálico/complicações , Tronco Encefálico/patologia , Hemangioma Cavernoso do Sistema Nervoso Central/complicações , Hemorragias Intracranianas/epidemiologia , Adulto , Neoplasias do Tronco Encefálico/patologia , Feminino , Seguimentos , Hemangioma Cavernoso do Sistema Nervoso Central/patologia , Humanos , Incidência , Hemorragias Intracranianas/etiologia , Hemorragias Intracranianas/patologia , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Fatores de Risco , Resultado do Tratamento , Adulto JovemRESUMO
OBJECT: The goal of this study was to evaluate surgical outcomes of pediatric brainstem cavernous malformations (CMs) and identify the risk factors associated with postoperative full recovery and rebleeding. METHODS: The clinical charts and radiographs from a series of 52 pediatric patients (37 male and 15 female; mean age 12.2 years; range 1-17 years) who underwent surgery for brainstem CMs between 1996 and 2011 were reviewed. Follow-up evaluation measures were obtained retrospectively. Neurological function was evaluated using the modified Rankin Scale (mRS) score. RESULTS: The lesion locations among the 52 patients included the midbrain (n = 7, 13.5%), pons (n = 38, 73.1%), and medulla (n = 7, 13.5%). The mean duration of symptoms was 18.5 months, and the preoperative annual hemorrhage and rebleeding rates were 12.3% and 32.5% per patient-year, respectively. The mean lesion size was 2.1 cm. Gross-total resection without surgery-related death was achieved in 49 patients (94.2%). Immediate postoperative reduced neurological function was observed in 17 patients (32.7%). Surgical morbidities developed in 25 patients (48.1%) and remained in 11 patients (21.2%) after 7.9 years of follow-up. The mean mRS scores at admission, discharge after surgery, 3 and 6 months postsurgery, and recent evaluation were 2.0, 2.3, 2.0, 1.5, and 1.0, respectively. The postoperative mRS scores at 6 months (p < 0.001) and on recent evaluation (p < 0.001) were significantly lower than those at admission. Postoperative rebleeding occurred in 2 patients, and the postoperative annual rebleeding rate was 0.5% per patient-year. By the most recent evaluation, 10 patients (19.2%) had achieved full recovery and all patients were either improved (n = 32, 61.5%) or unchanged (n = 20, 38.5%). The adverse predictors for full recovery included age ≥ 12 years (HR 0.230, p = 0.021), ≥ 2 preoperative hemorrhages (HR 0.124, p = 0.048), and poor preoperative status (HR 0.197, p = 0.040). An HR < 1 predicted poor complete recoveries. The single risk factor predicting postoperative rebleeding was incomplete resection (χ2 = 4.340, p = 0.037). CONCLUSIONS: Fair outcomes for pediatric brainstem CMs could be obtained through surgery, but only a few patients achieved full recovery. Thus, to minimize surgical morbidity, surgical planning must be tailored to individual patients in all cases in which an operation is warranted. Complete resection must be attempted to reduce the risk of postoperative rebleeding. The predictors associated with complete postoperative recovery were referential for determining treatment.
Assuntos
Tronco Encefálico/irrigação sanguínea , Tronco Encefálico/cirurgia , Hemorragia Cerebral/cirurgia , Hemangioma Cavernoso do Sistema Nervoso Central/fisiopatologia , Hemangioma Cavernoso do Sistema Nervoso Central/cirurgia , Recuperação de Função Fisiológica , Adolescente , Tronco Encefálico/fisiopatologia , Hemorragia Cerebral/etiologia , Hemorragia Cerebral/fisiopatologia , Criança , Pré-Escolar , China , Feminino , Hemangioma Cavernoso do Sistema Nervoso Central/complicações , Hemangioma Cavernoso do Sistema Nervoso Central/patologia , Humanos , Lactente , Estimativa de Kaplan-Meier , Masculino , Prontuários Médicos , Procedimentos Neurocirúrgicos/métodos , Procedimentos Neurocirúrgicos/normas , Recidiva , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Procedimentos Cirúrgicos Vasculares/métodos , Procedimentos Cirúrgicos Vasculares/normasRESUMO
OBJECT: The aim of this study was to investigate the clinical appearance of untreated pediatric brainstem cavernous malformations (CMs) and to identify the hemorrhage risks and functional outcomes. METHODS: All pediatric patients with a diagnosis of brainstem CM between 1985 and 2012 were registered. The clinical chart and radiographs were recorded, and follow-up evaluations were obtained prospectively. RESULTS: A total of 85 patients (69.4% male) were included with a mean age of 12.7 years. Sixty-seven patients (78.8%) had prior hemorrhage, and 6 patients (7.1%) were asymptomatic. There were 15 midbrain lesions, 53 pons lesions, and 17 medulla lesions. The mean lesion size was 1.9 cm. During a total of 401.6 patient-years of follow-up, 47 hemorrhages occurred in 37 patients, and the annual hemorrhage rate was 11.7% per patient-year. The mean hemorrhage interval was 47.8 months. The hemorrhage risk declined over time, especially after the first 2 years. Both a lesion size ≥ 2 cm (hazard ratio [HR] 2.122, p = 0.037) and the presence of perilesional edema (HR 2.192, p = 0.039) predicted future hemorrhage and were associated with a high annual hemorrhage rate. The hemorrhage-free survival at 6 months was 85.7%, and at 1, 5, 10, and 15 years was 71.5%, 49.4%, 27.5%, and 13.7%, respectively. At the most recent functional evaluation, 33 patients (38.8%) had improved, 32 (37.6%) had stabilized, and 20 (23.5%) had worsened, without any deaths. Twenty-two patients (25.9%) obtained a full recovery. Prospective hemorrhage (HR 0.191, p = 0.003) was the adverse predictor for full recovery. Full recovery primarily occurred within the first 12 months, after which the chance of full recovery decreased. The cumulative percentage of complete recovery at 6 months was 32.7%, and at 1, 3, and 5 years was 40.8%, 43.6%, and 49.2%, respectively. CONCLUSIONS: In this study the hemorrhage rate was relatively high in pediatric brainstem CMs, although the functional outcome was acceptable. The decline in hemorrhage risk and the identified adverse predictors in this study were helpful for clinicians and patients when deciding on treatment. Referral bias and the insufficient follow-up period of the study were highlighted as limitations.